In vivo biodistribution study of TAT-L-Sco2 fusion protein, developed as protein therapeutic for mitochondrial disorders attributed to SCO2 mutations
نویسندگان
چکیده
منابع مشابه
Human Sco1 and Sco2 function as copper-binding proteins.
The function of human Sco1 and Sco2 is shown to be dependent on copper ion binding. Expression of soluble domains of human Sco1 and Sco2 either in bacteria or the yeast cytoplasm resulted in the recovery of copper-containing proteins. The metallation of human Sco1, but not Sco2, when expressed in the yeast cytoplasm is dependent on the co-expression of human Cox17. Two conserved cysteines and a...
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a In recent years, graphene and transition metal dichalcogenides (TMDs) have been at the forefront of candidate materials for next-generation electronic devices. In this study, we will consider transition metal oxides (TMOs), which are a class of materials that can exist in two-dimensional geometries, but exhibit unique properties due to the strong correlation between electrons. Density functio...
متن کاملCytochrome c oxidase deficiency due to mutations in SCO2, encoding a mitochondrial copper-binding protein, is rescued by copper in human myoblasts.
Mutations in SCO2, a cytochrome c oxidase (COX) assembly gene, have been reported in nine infants with early onset fatal cardioencephalomyopathy and a severe COX deficiency in striated muscle. Studies on a yeast homolog have suggested that human Sco2 acts as a copper chaperone, transporting copper to the Cu(A) site on the Cox II subunit, but the mechanism of action remains unclear. To investiga...
متن کاملTissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1.
The biogenesis of eukaryotic COX (cytochrome c oxidase) requires several accessory proteins in addition to structural subunits and prosthetic groups. We have analysed the assembly state of COX and SCO2 protein levels in various tissues of six patients with mutations in SCO2 and SURF1. SCO2 is a copper-binding protein presumably involved in formation of the Cu(A) centre of the COX2 subunit. The ...
متن کاملAssociation of mutations in SCO2, a cytochrome c oxidase assembly gene, with early fetal lethality.
BACKGROUND SCO2 is a cytochrome c oxidase (COX) assembly gene that encodes a mitochondrial inner membrane protein that probably functions as a copper transporter. Mutations in SCO2 have been associated with severe COX deficiency and early-onset fatal infantile hypertrophic cardiomyopathy, encephalopathy, and neurogenic muscle atrophy. Fetal wastage has not been described in association with mut...
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ژورنال
عنوان ژورنال: Molecular Genetics and Metabolism Reports
سال: 2020
ISSN: 2214-4269
DOI: 10.1016/j.ymgmr.2020.100683